Assessment of hearing and speech comprehension - preventive examinations for children and adolescents with osteogenesis imperfecta.
- 1Department of Paediatrics, Newborn Pathology and Bone Metabolic Diseases, Medical University of Lodz, Lodz, Poland. [email protected].
- 2Department of Paediatrics, Newborn Pathology and Bone Metabolic Diseases, Medical University of Lodz, Lodz, Poland.
- 3Department of Paediatric Otorhinolaryngology, Medical University of Lodz, Lodz, Poland.
Abstract
Introduction
In children, acquired hearing loss typically occurs as a result of genetic disorders, ototoxic drugs and infections. It has also been noted as a serious complication of osteogenesis imperfecta (OI), a genetic connective tissue disorder that mainly arises from disorders of collagen type 1 synthesis; its other symptoms include multiple fractures and skeletal deformities, blue sclera, enamel hypoplasia and tooth fragility. To date, most studies on hearing loss in patients with OI have focussed on the adult population, due to the low occurrence among children. The aim of the study was to assess the level of hearing and speech comprehension in children and adolescents diagnosed with OI compared to a group without OI and without otological problems.Materials and methods
The study included a total of 103 children aged 5-17 years (54 boys and 49 girls). The patients were divided into two groups for the purpose of the study. Group I comprised 53 patients diagnosed with osteogenesis imperfecta (OI) type I and III, who were hospitalised at the Department of Paediatrics, Neonatal Pathology and Metabolic Bone Diseases between 2019 and 2025. Group II comprised 50 children who visited the Department of Paediatric Otorhinolaryngology for reasons unrelated to hearing disorders; this was used as the control group. A comprehensive auditory evaluation was conducted for all patients, encompassing an otoscopic examination, pure tone audiometry, speech audiometry, immittance audiometry, and otoacoustic emission (OAE) testing.Results
Compared to controls, Group I exhibited significantly poorer hearing thresholds (dB-decibel) in pure tone audiometry (PTA) at low and medium frequencies (250, 500, 1000, 2000 Hz-hertz) for both the right and left ears (p < 0.05). Similarly, patients with OI type III demonstrated a higher hearing threshold (dB) compared to type I (p < 0.05). Immittance audiometry revealed a smaller ear canal volume in Group I compared with controls (p < 0.05). The tympanic membrane was found to have lower compliance in OI type III compared to type I (p < 0.05) for both the right ear (RE) and left ear (LE). Group I also exhibited a higher speech detection and reception threshold compared to the control group (p < 0.05).Conclusions
In patients diagnosed with osteogenesis imperfecta (OI), the onset of hearing impairment may manifest at lower frequencies and be accompanied by a smaller external canal volume and reduction in tympanic membrane compliance. Pure tone audiometry, immittance audiometry and speech audiometry should be considered integral components of any comprehensive hearing monitoring programme. Children with OI may encounter difficulties in comprehending verbal communication in challenging acoustic environments. In such cases, preventative measures, systematic monitoring procedures and early intervention strategies should be considered to enhance mental, emotional and educational development, and quality of life, in children with OI.
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BibTeX
@article{wozniak2026assessment,
title = {{Assessment of hearing and speech comprehension - preventive examinations for children and adolescents with osteogenesis imperfecta.}},
author = {Elżbieta Woźniak and Bogumiła Górczewska and Ewa Kott and Magdalena Wacławek and Elżbieta Jakubowska-Pietkiewicz},
journal = {Head \& face medicine},
year = {2026},
month = jul,
doi = {10.1186/s13005-026-00643-9},
url = {https://europepmc.org/article/MED/42928283},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Assessment of hearing and speech comprehension - preventive examinations for children and adolescents with osteogenesis imperfecta.
AU - Elżbieta Woźniak
AU - Bogumiła Górczewska
AU - Ewa Kott
AU - Magdalena Wacławek
AU - Elżbieta Jakubowska-Pietkiewicz
PY - 2026
DA - 2026/07/01
JO - Head & face medicine
DO - 10.1186/s13005-026-00643-9
UR - https://europepmc.org/article/MED/42928283
AB - Introduction In children, acquired hearing loss typically occurs as a result of genetic disorders, ototoxic drugs and infections. It has also been noted as a serious complication of osteogenesis imperfecta (OI), a genetic connective tissue disorder that mainly arises from disorders of collagen type 1 synthesis; its other symptoms include multiple fractures and skeletal deformities, blue sclera, enamel hypoplasia and tooth fragility. To date, most studies on hearing loss in patients with OI have focussed on the adult population, due to the low occurrence among children. The aim of the study was to assess the level of hearing and speech comprehension in children and adolescents diagnosed with OI compared to a group without OI and without otological problems.Materials and methods The study included a total of 103 children aged 5-17 years (54 boys and 49 girls). The patients were divided into two groups for the purpose of the study. Group I comprised 53 patients diagnosed with osteogenesis imperfecta (OI) type I and III, who were hospitalised at the Department of Paediatrics, Neonatal Pathology and Metabolic Bone Diseases between 2019 and 2025. Group II comprised 50 children who visited the Department of Paediatric Otorhinolaryngology for reasons unrelated to hearing disorders; this was used as the control group. A comprehensive auditory evaluation was conducted for all patients, encompassing an otoscopic examination, pure tone audiometry, speech audiometry, immittance audiometry, and otoacoustic emission (OAE) testing.Results Compared to controls, Group I exhibited significantly poorer hearing thresholds (dB-decibel) in pure tone audiometry (PTA) at low and medium frequencies (250, 500, 1000, 2000 Hz-hertz) for both the right and left ears (p < 0.05). Similarly, patients with OI type III demonstrated a higher hearing threshold (dB) compared to type I (p < 0.05). Immittance audiometry revealed a smaller ear canal volume in Group I compared with controls (p < 0.05). The tympanic membrane was found to have lower compliance in OI type III compared to type I (p < 0.05) for both the right ear (RE) and left ear (LE). Group I also exhibited a higher speech detection and reception threshold compared to the control group (p < 0.05).Conclusions In patients diagnosed with osteogenesis imperfecta (OI), the onset of hearing impairment may manifest at lower frequencies and be accompanied by a smaller external canal volume and reduction in tympanic membrane compliance. Pure tone audiometry, immittance audiometry and speech audiometry should be considered integral components of any comprehensive hearing monitoring programme. Children with OI may encounter difficulties in comprehending verbal communication in challenging acoustic environments. In such cases, preventative measures, systematic monitoring procedures and early intervention strategies should be considered to enhance mental, emotional and educational development, and quality of life, in children with OI.
SN - 1746-160X
ER -